Disorders of the Eye

Nelson Self Assessments website 17th Edition

Question . 1. Delayed removal of a congenital and complete unilateral cataract may

lead to:

A. Amblyopia
Explanation: Amblyopia is a vision loss that is due not to a specific organic
lesion but rather to deprivation or disuse of the retina. Even after delayed
removal of a cataract or correction of strabismus, the previously unused retina
"tunes out" the image. (See Chapter 619 in Nelson Textbook of Pediatrics,
17th ed.)
B. Glaucoma
C. Uveitis
D. Strabismus
E. Nyctalopia

Question . 2. Cataracts are noted in all of the following Except:

A. Rubella (congenital)
B. Galactosemia
C. Galactokinase deficiency
D. Neonatal hypoglycemia
E. Hypocalcemia
F. Lowe syndrome
G. Hyperoxygenation
Explanation: Hyperoxia in preterm neonates usually causes retinopathy of
prematurity, which occasionally is associated with cataracts. Oxygen does not
directly cause cataracts. (See Chapter 619 in Nelson Textbook of Pediatrics,
17th ed.)
H. Steroid therapy
I. Child abuse

Question . 3. An 18-mo-old manifests pendular nystagmus, head nodding, and

torticollis. Findings on a cranial MRI scan are normal. This child is most likely to
have:

A. Epilepsy
B. Congenital blindness
C. Neuroblastoma
D. Dysmetria
E. Spasmus nutans
Explanation: The acquired triad of nystagmus, head nodding, and torticollis, in
its classic form, is self-limited and benign. Nonetheless, children with brain
tumors may have signs resembling components of spasmus nutans. (See
Chapter 614 in Nelson Textbook of Pediatrics, 17th ed.)

Disorders of the Eye - Nelson Self Assessments website 17th Edition 1

Question . 4. A 7-yr-old girl experiences fullness of the right upper eyelid and
downward displacement of the eye over a 2-mo period. The right eye also appears to
be proptotic. Which of the following is the most likely diagnosis?
A. Myasthenia gravis
B. Right superior oblique palsy
C. Chalazion
D. Rhabdomyosarcoma
Explanation: Rhabdomyosarcoma of the face, orbit, and sinus often presents
early because of the space-occupying and displacement effects of tumor
growth. (See Chapter 624 in Nelson Textbook of Pediatrics, 17th ed.)
E. Hypothyroidism

Question . 5. The most common presenting sign of retinoblastoma is:

A. Heterophoria
B. Hypopyon
C. Leukocoria
Explanation: The clinical manifestations of retinoblastoma vary, but the initial
sign in the majority of cases is leukocoria (white pupillary reflex, also known
as "cat eye") instead of the normal red pupillary reflex. (See Chapter 621)
D. Coloboma
E. Red reflex

Question . 6. A 17-yr-old girl taking oral contraceptives presents with headache,

nausea, and vomiting. Physical examination reveals papilledema. The most
appropriate action is:
A. Discontinue the oral contraceptives and re-evaluate in 7-14 days
B. Discontinue the oral contraceptives and administer promethazine (Phenergan)
as needed for nausea and vomiting
C. Discontinue the oral contraceptives and administer oral corticosteroids
D. Perform computed tomography (CT) or magnetic resonance imaging (MRI)
study of the head
Explanation: Papilledema constitutes a neurologic emergency. Neuroimaging
should be performed, and if no intracranial masses are found, a lumbar
puncture for determination of cerebrospinal fluid pressure should be
performed. This patient may have pseudotumor cerebri. (See Chapter 622)
E. Perform lumbar puncture for determination of cerebrospinal fluid pressure

Question . 7. A 1-yr-old child presents with increased size of the cornea. Review of
systems reveals history of increased tearing and apparent sensitivity to light. The
cornea appears cloudy. The most likely diagnosis is:
A. Retinoblastoma
B. Glaucoma
Explanation: Symptoms of infantile glaucoma (glaucoma that begins in the
first 3 years of life) include the classic triad of epiphora (tearing), photophobia
(sensitivity to light), and blepharospasm (eyelid squeezing), which are usually
attributed to corneal irritation. An increase in intraocular pressure leads to
expansion of the globe, including the cornea. (See Chapter 623 in Nelson)
C. Chorioretinitis
D. Aniridia
E. Coloboma

Disorders of the Eye - Nelson Self Assessments website 17th Edition 2

Question . 8. Which of the following is the most appropriate initial treatment for the
child described in Question 9?

A. Topical glaucoma medications

B. Oral glaucoma medications
C. Corticosteroids
D. Surgery
Explanation: Unlike glaucoma in adults, in which medications are the first line
of therapy, the treatment of infantile glaucoma is primarily surgical.
Procedures used to treat glaucoma in children include surgery to establish a
more normal anterior chamber angle (goniotomy and trabeculotomy), to create
a route for aqueous fluid to exit the eye (trabeculectomy and seton surgery), or
to reduce aqueous fluid production (cyclocryotherapy and photocyclo-
coagulation). In many children, several operations are required to lower and
maintain the intraocular pressure adequately. Long-term medications may be
necessary as well. (See Chapter 623 in Nelson Pediatrics, 17th ed.)
E. Medications and surgery

Question . 9. A 12-yr-old boy has a small corneal abrasion detected by Wood lamp
examination. Which of the following is the most appropriate treatment?

A. Topical antibiotic ointment

Explanation: Corneal abrasions are treated with frequent applications of a
topical antibiotic ointment until the epithelium is completely healed. The use
of a semi-pressure patch does not improve healing time or decrease pain.
Furthermore, an improperly applied patch may itself abrade the cornea. A
topical cycloplegic agent (cyclopentolate hydrochloride 1%) can relieve the
pain from ciliary spasm in patients with large abrasions. Topical anesthetics
should not be given at home because they retard epithelial healing and inhibit
the natural blinking reflex. (See Chapter 625 in Nelson Pediatrics, 17th ed.)
B. Topical antibiotic ointment and a semipressure patch
C. Topical antibiotic ointment, a semipressure patch, and a topical cycloplegic
agent
D. Topical antibiotic ointment, a semipressure patch, and a topical anesthetic as
necessary for pain
E. Topical antibiotic ointment, a topical cycloplegic agent, and a topical
anesthetic as necessary for pain

Question . 10. All of the following statements concerning the newborn eye are correct
Except:

A. The cornea is smaller than that of an adult

B. Tears are often not present with crying until 1-3 mo of age
C. Ocular alignment may not be perfect in the first several months of life
D. Most infants are myopic (nearsighted)
Explanation: Most infants are hyperopic (farsighted). The cornea is smaller in
infants and grows to reach adult size near the age of 2 yr. Tears are not usually
produced with crying until a few months of age. It is common for children to
have strabismus in the first few months of life. (See Chapter 609 in Nelson)
E. Iris color may change in the first several months of life

Disorders of the Eye - Nelson Self Assessments website 17th Edition 3

Question . 11. Which of the following tests is not part of a routine eye examination?

A. Visual acuity
B. Pupil assessment
C. Preferential looking test
Explanation: Preferential looking tests are done by experienced examiners and
are not part of a routine eye examination. (See Chapter 610 in Nelson)
D. External examination
E. Ocular motility

Question . 12. Which of the following statements concerning abnormalities of

refraction is correct?

A. Children with hyperopia will often squint to see better

B. Astigmatism may be caused by an irregular shape of the cornea
Explanation: Astigmatism is commonly caused by an irregular corneal shape.
Children with hyperopia will accommodate to see better. Children with
myopia will frequently squint to see better. Scientific studies have not proved
that bifocals can cure myopia. Anisometropia occurs when there is a
difference in refractive error between the two eyes. It may lead to amblyopia
and decrease vision in one eye. However, the term anisometropia does not
indicate a difference in vision by itself. High levels of myopia may lead to
retinal detachment. (See Chapter 611 in Nelson Textbook Pediatrics, 17th ed.)
C. Myopia can be cured with the use of bifocal lenses
D. Anisometropia occurs when one eye sees better than the other
E. Myopia is always a benign condition

Question . 13. A 4-yr-old girl presents with a large esotropia and poor vision in one
eye. Both eyes are normal on examination. What is the most likely reason for her poor
vision?

A. Need for glasses

B. Malingering
C. Cataract
D. Suppression
E. Amblyopia
Explanation: This child probably has amblyopia secondary to her strabismus.
Although the need for glasses in only one eye or a cataract in one eye could
present in the same way, these problems are far less common in this age
group. (See Chapter 612 in Nelson Textbook of Pediatrics, 17th ed.)

Disorders of the Eye - Nelson Self Assessments website 17th Edition 4

Question . 14. A newborn is presented with what appear to be large pupils that do not
constrict to light. There is no family history of similar ocular findings. An appropriate
study to be performed in the future is:

A. CT scan of the head and orbit

B. Visual evoked potentials
C. Renal ultrasound study
Explanation: In aniridia, the pupils look large and nonreactive. Because
children so affected are at risk for Wilms tumor, renal ultrasound study is
indicated. (See Chapter 613 in Nelson Textbook of Pediatrics, 17th ed.)
D. Lumbar puncture
E. Cocaine test

Question . 15. A 2-yr-old boy presents with pronounced crossing of his left eye of 2
months' duration. On further examination, he is found to be excessively farsighted for
his age. The most likely diagnosis is:

A. Congenital superior oblique palsy

B. Accommodative esotropia
Explanation: Accommodative esotropia causes a crossing of the eyes
secondary to a high degree of farsightedness. Congenital esotropia does not
develop at this age. (See Chapter 614 in Nelson Textbook Pediatrics, 17th ed.)
C. Intermittent exotropia
D. Duane syndrome
E. Congenital esotropia

Question . 16. Which of the following statements concerning congenital ptosis is

true?
A. Surgery should be performed shortly after birth in all cases
B. Amblyopia will occur only if the lid covers the pupil
C. Amblyopia cannot occur once the lid is surgically elevated
D. Ptosis may occur in association with other ocular or systemic disorders
Explanation: Ptosis is often associated with other systemic disorders. Surgery
does not need to be performed early if the vision is developing normally.
Amblyopia can occur if the ptosis causes a unilateral astigmatism. It can occur
even if the pupil is not completely occluded. Amblyopia can continue to occur
even after successful ptosis surgery. Frontalis suspension is used for cases of
severe ptosis. (See Chapter 615 in Nelson Textbook of Pediatrics, 17th ed.)
E. Frontalis suspension surgery is used for mild cases of ptosis

Question . 17. A 3-mo-old girl is presented with tearing and mucous discharge from
her left eye that has been present since near birth. Her external examination is
otherwise normal. Appropriate forms of treatment would include:
A. Nasolacrimal massage
B. Topical antibiotics for infections if they occur
C. Cleansing of the lids
D. Probing of the nasolacrimal duct if the symptoms do not resolve with time
E. All of the above
Explanation: All of the above are correct. Many cases of nasolacrimal duct
obstruction will resolve within the first year of life. (See Chapter 616)

Disorders of the Eye - Nelson Self Assessments website 17th Edition 5

Question . 18. A 2-day-old infant is presented with bilateral conjunctival redness and
purulent discharge. The most likely diagnosis is:

A. Nasolacrimal duct obstruction

B. Ophthalmia neonatorum
Explanation: Ophthalmia neonatorum occurs shortly after birth. A
nasolacrimal duct obstruction does not usually cause significant conjunctival
redness this early. Congenital glaucoma is often associated with tearing but
not with a purulent discharge. Blepharitis and vernal conjunctivitis do not
generally occur at this age. (See Chapter 617 in Nelson Pediatrics, 17th ed.)
C. Congenital glaucoma
D. Blepharitis
E. Vernal conjunctivitis

Question . 19. All of the following disorders may present as a congenital corneal
opacity Except:

A. Sclerocornea
B. Congenital cataract
Explanation: A cataract is opacity of the lens, not the cornea. (See Chapter 618
in Nelson Textbook of Pediatrics, 17th ed.)
C. Infantile glaucoma
D. Peters anomaly
E. Epibulbar dermoid

Question . 20. A child presents with a dense unilateral cataract. All of the following
are important factors in the visual prognosis following the removal of the cataract
Except:
A. Whether a contact lens or an intraocular lens implant is used
Explanation: The mode of visual rehabilitation is not the most important factor
in the outcome of pediatric cataract surgery. Early diagnosis, age at time of
cataract development, and success with occlusion therapy are the most
important factors. (See Chapter 619 in Nelson Textbook of Pediatrics, 17th
ed.)
B. The compliance with patching for amblyopia
C. The length of time the cataract was present
D. The age of the child at presentation
E. The health status of the posterior segment of the eye

Question . 21. A young girl is found to have bilateral iritis on routine examination. On
further questioning, it is found that she has been limping for the last several weeks.
The most likely diagnosis is:

A. Sympathetic ophthalmia
B. Juvenile rheumatoid arthritis
Explanation: Uveitis in patients with JRA commonly presents without pain or
redness. Sympathetic ophthalmia is unilateral and occurs in the uninjured eye
following a significant ocular injury. Herpes simplex may cause an anterior
uveitis but is usually unilateral and associated with pain. Toxoplasmosis

Disorders of the Eye - Nelson Self Assessments website 17th Edition 6

 causes a posterior uveitis and is usually unilateral. Kawasaki disease is not
associated with arthritis and usually causes conjunctivitis. (See Chapter 620 )
C. Herpes simplex
D. Toxoplasmosis
E. Kawasaki disease

Question . 22. A 20-mo-old girl is noted by her parents to have a unilateral white
pupil. Funduscopic examination reveals a very large white mass that fills most of the
eye. All of the following statements regarding this patient are correct Except:

A. Genetic counseling is indicated for this family

B. Removal of the eye may be required
C. A CT scan may demonstrate calcium within the lesion
D. A biopsy should be performed as soon as possible
Explanation: This patient is most likely to have a retinoblastoma. Because it
can be a hereditary tumor, genetic counseling is indicated. Treatment of a
large unilateral tumor may require enucleation. Appropriate work-up may
include a CT scan to demonstrate calcium within the eye. Examination of the
other eye for smaller tumors should always be performed. Biopsy of the
suspected tumor is contraindicated, as it may lead to spread of the tumor cells.
(See Chapter 621 in Nelson Textbook of Pediatrics, 17th ed.)
E. Examination of the other eye should be performed with the patient under
anesthesia

Question . 23. A 4-yr-old boy who is being followed by his primary care doctor for
short stature is found to have poor vision in one eye. On ophthalmic examination, he
is noted to have an afferent pupillary defect on the affected side. His anterior segment
is normal, and his vision cannot be improved with glasses.
The most likely diagnosis is:
A. Strabismus
B. Cataract
C. Optic nerve hypoplasia
Explanation: Optic nerve hypoplasia may be associated with growth hormone
deficiency and poor vision. Patients with unilateral optic nerve abnormalities
will demonstrate an afferent pupillary defect. (See Chapter 622 in Nelson)
D. Glaucoma
E. Retinal detachment

Question . 24. A 3-mo-old boy is brought in by his parents because he appears to be

very light sensitive. On examination, he is tearing, and his corneas appear large and
cloudy. The most likely diagnosis is:
A. Infantile glaucoma
Explanation: The classic findings in infantile glaucoma are enlarged corneas,
cloudy corneas, tearing, and light sensitivity. Although a tear duct obstruction
also causes tearing, it does not cause a change in the size or clarity of the
cornea, and it is not associated with photophobia. (See Chapter 623 in Nelson)
B. Nasolacrimal tear duct obstruction
C. Peters anomaly
D. Infantile cataracts
E. Uveitis

Disorders of the Eye - Nelson Self Assessments website 17th Edition 7

Question . 25. A 2-yr-old child presents with a 1-day history of unilateral proptosis
and fever. Appropriate next steps in the diagnosis and treatment of this child could
include all of the following Except:

A. CT scan of the head, orbit, and sinuses

B. Ophthalmologic consultation
C. Administration of a broad-spectrum antibiotic
D. Surgical drainage of any abscess
E. Determination of TSH and TRH blood levels
Explanation: Although thyroid disease may manifest with unilateral proptosis,
it is not associated with fever and does not usually develop this rapidly. The
other answers are appropriate steps in the management of a child with a
preseptal or orbital cellulitis. (See Chapter 624 in Nelson Pediatrics, 17th ed.)

Question . 26. Which of the following statements regarding children with traumatic
hyphemas is most correct?

A. This is generally a benign condition

B. If secondary bleeding does not occur by the second day, there is little risk of
further complications
C. Children with sickle cell disease are at increased risk for loss of vision
Explanation: Children with sickle cell disease are at increased risk for vision
loss following a traumatic hyphema. Traumatic hyphemas can be a significant
cause for vision loss. Rebleeding is most common 3 to 5 days after the initial
injury. Blood within the anterior chamber can lead to corneal blood staining
and/or glaucoma. Patients with a history of a hyphema are at risk for the
development of glaucoma later in life. (See Chapter 625 in Nelson Textbook
of Pediatrics, 17th ed.)
D. Once the blood leaves the anterior chamber, there is no longer any risk from
the injury causing the hyphema
E. The blood itself produces no ill effects

Disorders of the Eye - Nelson Self Assessments website 17th Edition 8